Case Presentation: A 26-year-old healthy right-handed female presented to the hospital with a sudden onset of right arm swelling and discoloration. She was visiting the US from Australia and flew about two weeks prior. On the day of presentation, she was shopping when she noticed right arm pain, swelling, and discoloration. She did not suffer any recent trauma. She had an abortion about 8 weeks prior and since then had restarted taking oral contraceptives. She otherwise is very active, and is an avid hiker. In the ER, D-dimer was elevated at 781. Upper extremity ultrasound showed occlusive right subclavian thrombus extending to the axillary vein and upper cephalic vein. She also reported having episodes of shortness of breath a few days after she arrived, so chest CT was done which showed bilateral lower lobe segmental and subsegmental pulmonary emboli. She was started on a heparin drip for treatment. Interventional radiology was consulted given concern for Paget-Schroetter’s syndrome and need for possible thrombolysis. After reviewing the CT scan and ultrasound imaging, IR agreed her presentation was most consistent with Paget-Schroetter’s syndrome, with likely external compression of her subclavian vein. Their recommended treatment was continued anticoagulation and thrombolysis. After a long discussion with the patient, she ultimately decided on continuing anticoagulation with xarelto and outpatient follow up with interventional radiology for thrombolysis back home in Australia.
Discussion: Paget-Schroetter’s Syndrome, also known as “effort” thrombosis, is a rare condition characterized by the spontaneous development of an upper extremity subclavian and axillary DVT. Anatomic abnormalities at the thoracic outlet where the subclavian vein passes between the first rib and clavicle, as well as repetitive upper extremity movements, cause trauma to the subclavian vein endothelium resulting in an inflammatory response and clot formation. Paget-Schroetter’s is most commonly seen in young, healthy and athletic individuals and symptoms usually start after an inciting event. Patients frequently present with sudden onset of swelling, blue discoloration, heaviness, and pain in the arm. Diagnosis can be made initially by Duplex or Doppler ultrasound as it is noninvasive and inexpensive. The gold standard for diagnosis is via catheter-based venography, which is usually done if suspicion remains high despite equivocal or negative noninvasive studies. Aggressive treatment with anticoagulation, catheter-directed thrombolysis, and potentially surgical decompression of the thoracic outlet are important in preventing further complications including pulmonary embolism, post thrombotic syndrome and recurrent thrombosis.
Conclusions: Paget-Schroetter’s syndrome is a rare condition that should be considered in younger individuals who develop sudden onset of an upper extremity DVT. Early recognition, diagnosis, and treatment with a multimodal approach are essential in avoiding serious and debilitating long term complications.